Things you might like to know...

We lasted 18 days at home before a return to hospital....I have been putting off updating this blog because I wanted to write that we were back home, which we are now, but also because managing the needs of four children during the school holidays and a husband who is working freelance means that there are even less minutes, let alone hours in the day to update. In not writing for a while I discovered how many people actually read this blog as so many people have contacted me to ask me to update it. It is so flattering to know people enjoy my writing and I promise to try harder to update a bit more frequently. In fact it will make my life easier to do so as so much has happened since my last post that it becomes it nighmare to condense it all into a manageable chunk of text for people to read, so in advance, apologies if this one rambles a bit but here we go.......

I looked back over my last post and the signs were all there - the early return from Chase with high temp...of course it was not just going to go away... The day after our return from Chase Daisy was due for bloods so we decided to run some blood cultures also to check that there was no line infection, I think 18 days at home had made me forget too quickly as I was caught off guard when a call came through from the ward to say that the cultures had come through as positive and could I bring Daisy in to start IV antibiotics.....

Hindsight is such a great thing isn't it - she had been looking increasingly pale as the days at home had worn on and I think I was just trying to ignore it. Things went well in hospital for a while, she responded to antibiotics and the plan was for me to take her home and continue to administer IV antibiotics at home, but of course this was never going to be a smooth journey. To cut a long story short, big sister Xanthe accidentally pulled out Daisy's jejunostomy tube (all 30cms of it, sutured into place in her intestine) while Daisy was allowed home for a few hours before discharge. I had been out at the time and came back to chaos - Daisy screaming, Theo panicking, Xanthe very tearful and Andy with a 30 cm jejunostomy tube in his hands. Normally if a child just has a gastrostomy button, which Daisy used to have, it is possible to simply reinsert it - we have done this at home previously. A jejunostomy is a different matter as it has to be put back surgically....and so our problems began.

As far as GOS was concerned Daisy was not an emergency as she had a Hickman line and could receive nutrition via TPN, the fact that we had to run her TPN over 24 hours and switch her immunosuppressants back to IV infusions lasting for 2 hours twice a day, didn't seem to matter. We were frantically trying to get her back into GOS to get her jej tube back in as she had been so close to home after what had initially been just a blip. But of course once one thing goes wrong the house of cards just falls down, the fluid came back on Daisy's hips causing her massive pain and then no suprises she spiked another temperature. Of course Daisy couldn't spike a temperature in a safe controlled environment like the hospital ward, instead she waited until we had been allowed a few hours day release at Chase Hospice.

The plan had been to leave Daisy with Gemma and Rachel at Chase while I took the three other children for lunch. We had just settled down for a lovely lunch when I got a call from Gemma - Daisy's temp was up and she had become poorly - all in the space of an hour. Cue a frantic request to our waitress for takeaway containers, our meals were poured into them and we ran through the streets of Guildford. The children were so brilliant, taking it all in their stride as always. I was doing my quick mental scenarios of what to do - if she was dangerously septic then it was a 999 call scenario, if not well enough to travel back to Queen Mary's then we would have to take her to the nearest hospital, Royal Surrey, who not suprisingly have their own set of notes on Daisy. Fortunately when I got to Chase I felt I had enough time to get her back to Queen Marys. The lovely girls from Chase dealt with the issue of what to do with the other children by taking them swimming and arranging to bring them home separately.

Daisy was started back on IV antibiotics, yet again but at least GOS had come through with a date for the surgery. The biggest issue with having Daisy in over the summer holidays was not only that the children were on holiday but also anti- Swine flu measures meant that only two named adults could be with Daisy on the ward so I was left juggling children, Andy's work schedule and Daisy on 24 hour TPN. The agreement with GOS when we had been discharged had been that if Daisy ended back up in hospital again only Andy or I would access her hickman line. That worked well for the first few days, but as the days turned into weeks and the number of times her line needed to be accessed increased with infection the practicalities of this became more and more complex. The bottom line was that I was expected to be two places at once, 24 hours a day with Daisy administering 4 hours worth of IV infusions plus various antibiotics, taking bloods and managing her TPN as well as looking after our 3 other children on their summer holiday - clearly not practical especially as all my friends were on holiday (and why should they have to look after another child during the school holidays), by this stage all the holiday clubs were booked up and my mother had swine flu and couldn't come up. At one point I even had to leave the children for over an hour in the lift lobby outside the hospital ward door playing on their nintendo's while I sorted Daisy out! A complete nightmare!

I think our experience of looking after Daisy over the summer in the hospital shows that with Daisy you can have plans - for example being strict on exactly who can touch her hickman line, but the reality of our lives means that these plans need to be fluid. As I said to the Matron of the Children's ward would she expect a member of her staff to work 24 hours a day, seven days a week with no break? I think what really brought it home for me also was when Daisy developed an infection while on the ward and I was worried about her - I wanted to be worried about her as a mum, not also worrying about managing the medical side of her. The pressure of managing a lot more of Daisy's medical needs while she was an inpatient knocked both Andy's and my confidence as we realised how much pressure there is on managing Daisy's complex medical needs. The GOS policy is to minimise the number of people accessing a TPN dependent child's central line in order to prevent infection, but the reality is this needs to be balanced with the practical and emotional needs of the family. We are going to have further conversations with all of the teams involved in Daisy's nursing care to fine tune plans based on our experiences over the summer.

Anyway, to cut a long story short, Daisy ended up back on Rainforest ward and eventually she had her jejunostomy replaced. Having spent 2 weeks with just a naso-gastric tube holding the stoma open we were not even sure if the existing stoma would be viable. This was pointed out to me by the surgeon who came out of the theatre to inform me that if they could not use the existing stoma they would have to wake Daisy up and rebook her as they did not have enough time to do the more complicated procedure of opening a new stoma. Well, thankfully (and I say thankfully for the surgeon's sake as she seemed to think it would be OK to anaesthetise my child for possibly no reason) the stoma was viable and she came back from theatre with a lovely shiny new trans-gastric jejunostomy.....and an allergic rash to the anaesthetic which required IV antihistamine...

While we were in GOS Daisy had a scheduled appointment with her orthopaedic surgeon. This has to rate as the worst meeting with a consultant I have ever had, for so many reasons. At the time Andy was at home with a bad kidney infection, running a high temperature and trying to look after 3 children, I was completely sleep deprived and emotionally raw . Orthopaedic clinics are always chaotic and this was no exception, lots of wheelchairs means limited space, ontop of that we were late and Daisy's infusion had not finished, I forgot to pick up her notes when I left the ward so that caused further delays. When we got to the surgeon he did not even acknowledge Daisy and just leapt straight into "well you want me to look at her hips" - well no actually I would like you to understand some of the complex orthopaedic issues the children with costello syndrome have, currently manifesting as severely tightened achilles tendons causing her an inability to put her feet flat on the ground or walk unaided, her risk of scoliosis, and yes, the fact that she keeps getting this fluid on her hips which is not a costello thing and is causing her a lot of pain.....that's what I wanted to say but instead I blubbed some nonsense and burst into tears, something I never do infront of health professionals. It probably helped slightly as, although he still did not interact with Daisy (I guess this is true of most surgeons, they are used to seeing their patients asleep), he said her feet needed surgery and that her hips were a rheumotology issue and the gastro team on Rainforest should involved rheumotology in her care. Bang, consultation over, except it was only later I realised there was no plan for scheduling her foot surgery and the problem with Daisy's hips was not his problem....

We waited all day on Rainforest for the rheumotology team to come and review Daisy, possibly with the plan of booking her in to inject steroids into her hips under general anaesthetic. The plan had been to transfer back to Queen Mary's that day so transport was delayed until this review, the review that never came because at 5pm a call came through to the ward that the Rheumotology registrar could not make it that day (something to do with being the Friday before the bank holiday weekend perhaps) and our local hospital should refer and sort it out - bloody great. So there we were, no ambulances available to transport Daisy back to Queen Marys and no further forward on sorting out the problem with her hips...we got back to Queen Mary's at 9.30pm and as for the Rheumotology appointment, well we are still waiting, I'm planning to see what tomorrow's post brings and then it may be toys out of the pram time again.

The rest of Daisy's time in hospital was spent weaning her TPN down from 24 hours a day back to 14 hours and building up her jejunostomy feeds ml by ml to 15mls an hours for 10 hours a day - no hopes of any breaks from some sort of pump yet as her blood sugar drops as soon as they are stopped. But the good news is that after a month in hospital - in fact adding it up it was 12 months in hospital altogether with just 18 days at home - Daisy came back home again last Tuesday!!! Yippeeee!

We are seizing the moment as we strongly expect another crisis to hit, there is no reason not to expect this, so in the meantime while she is looking the best she has for months, she is back at school for 4 hours a day. And loving every minute of it of course. We are just loving having her home - everything is so much easier when she is at home; updating the blog, doing the TPN, doing the meds, helping the other children with their homework, catching up with my friends, spending time with my husband - it's just great and I really do savour these moments to keep me going during the inevitable hospital stays that will come.

Next week brings a cardiology appointment and the worry that brings with it that her cardiomyopathy may be worse still. But it also brings the normality of family life as the children get back into the full swing of school life, after school clubs, music lessons, swimming. With Daisy at school during the morning I can start attacking the cupboards which have been stuffed full of clutter and papers over the past year - the obsessive compulsive in me is looking forward to this greatly!!!!

Daisy has now been home for 2 weeks and, just like the pain of childbirth, memories of the endless months in hospital are slowly disappearing.... Just because Daisy is home does not mean she is better and the stress and worry of looking after her is still there, however this time we are in control and at least we are in our own environment.

The first week at home was spent getting into the swing of doing the TPN at home, clearly 10 months in hospital has institutionalised me as I have arranged Daisy's drug cupboard like a hospital cupboard and labelled all the shelves and boxes , sometimes the obsessive compulsive streak in me can be quite handy. We have now been trying to arrange and organise everything Daisy needs to have some sort of normality and for Andy and I to get a break. We have been lucky in getting our care package hours increased to get some more support during the day and we have relented and agreed to having night support which will mean having someone in the house for a few nights a week to manage Daisy's needs overnight. We have always shied away from this sort of support, preferring to rely on time spent at our hospice as overnight help, but as Daisy has moved the goalposts in her needs somewhat we have to accept more help with her. Of course this is all in the planning stage - now we need to get the right people in place to help us. When you have a child with complex needs you get used to having people come to the house all the time, over the years we have got used to a regular team of people - our friends from Chase Hospice and The Rainbow Trust, community nurses, key worker, social worker, physio. Now our team will be expanded even more.

We had a discharge videoconference with the Great Ormond Street Team and Queen Marys team a few days after coming home. Some people couldn't make the meeting but I was still amazed with how many people are involved in Daisy's medical care - and this was only to discuss the gastro elements of her life. We now have plans for managing future line infections and how to manage various other emergencies. The main plan to stay infection free however is for only Andy or I to ever touch Daisy's hickman line. This is a big emphasis with the GOSH home TPN programme, their research shows that the less people accessing the line, the less risk of infection. We also have to use anti-microbial "line locks" which have been proven to decrease infections in home TPN patients by 70%. The bottom line is to try and optimise Daisy's chances of remaining infection free we have to commit to only being the ones to put up and take down the TPN, take bloods from her line and do all the other stuff involved with managing a child with a central line on home TPN. At times over the past 10 months we were very close to not bringing Daisy home alive so this is a small price to pay for having her home.

Our second week at home has been spent at our wonderful hospice, Chase, in Guildford. Daisy stayed in her own room (complete with obligatory TV showing continuous Peppa Pig episodes) while the three other children, Andy and I stayed in an upstairs flat in the building. I did the TPN connections in the evenings and disconnections in the morning but apart from answering questions on Daisy's care my role was redundant which meant I could indulge in some retail therapy in Guildford and enjoy lovely walks along the canal near the hospice with Andy and the children. In fact tempting the children out into the fresh air for walks was really difficult as there is so much to do at Chase and the british weather even managed to shine for a couple of days so they could play in the garden. We left on Sunday, a bit worried as Daisy decided to spike a temperature and I had visions of putting the A&E plan into action. Her temp came down later in the evening and I am keeping my fingers crossed it was nothing sinister, although we did blood cultures this morning to rule out any infection. Daisy is massively at risk of infection at the moment as not only is she immunsuppressed because of the treatment for the inflammatory bowel disease, but she is also neutropenic, which means she does not have enough of the disease fighting white blood cells to cope with an infection. The GOS doctors think this may be a side effect of one of her medications, unfortunately this medication is working well to help calm down her inflammation so they don't want to take her off it, so for the timebeing she is having regular bloods done to keep a close eye on things.

We have a week at home now and I am still trying to get to the bottom of the mountain of clutter that has grown in all the cupboards since Daisy has been born. The best thing is just being able to spend family time together , Daisy's infection risk means that we can't plan too much but it's so nice all be under one roof - we regularly ignore the phone (sorry to all those that have experienced this) and the door bell because after months of having people around us all the time, it's just so good to be just us....

I have four extraordinary children, my two boys have a diagnosis of high functioning autism yet they could not be more different, my eldest daughter is a creative social justice warrior, my youngest daughter was born with a very rare genetic condition.

In December 2015 I also became a widow, my husband, the father of my children & partner of 27 years, Andy, died of cancer, leaving us with a gaping hole in our lives. 13 months later our darling daughter Daisy died.

My life was never going to be predictablebut as Andy always said, it is what it is, it's how you respond which makes the difference.